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ABSTRACT A-T is an autosomal-recessive syndrome characterized by neurologic abnormalities, variable ID, and a 10% prevalence rate of cancer at a mean age of 12.5 yrs. A-T patients have

excess proportions of lymphoid tumors and selected carcinomas compared to types in the general population. A-T lymphomas resemble nonlymph-oblastic childhood forms and differ from the

immunoblastic sarcomas prevalent in Wiskott-Aldrich syndrome and combined ID. To test the hypothesis that ID predisposes to cancer in A-T, particularly to lymphoma, we selected cases in

which serum IgA, and/or mitogen assays and skin tests were measured prior to or at cancer dx. Immune function test results were available on 1/3 of ICR cases (36/108). Malignancies by type

were lymphoma (44%), leukemias (22%), Hodgkin's disease (8%), and carcinomas (25%); these proportions were not different from that observed for the other 72 cases (X2 =.684;

1>P>.1). The prevalence of -/↓ IgA and/or ↓ cell-mediated immunity (CMI) among these 36 patients paralleled the immune function patterns abstracted from detailed literature reports for

57 A-T patients without cancer. A-T cases with both -/↓ IgA and ↓ CMI developed carcinoma and non-Hodgkin's lymphomas with similar frequency (29%, 36%, respectively). This

retrospective analysis suggests that defects of humoral and/or CMI do not alone predict susceptibility to lymphoma or other cancer in A-T. Supported by USPHS CP-43384. ARTICLE PDF AUTHOR

INFORMATION AUTHORS AND AFFILIATIONS * U. MN Med. Sch., Depts. Peds. & Lab. Med./Path., Mpls Alexandra H Filipovich, Beatrice P Spector & John H Kersey Authors * Alexandra H

Filipovich View author publications You can also search for this author inPubMed Google Scholar * Beatrice P Spector View author publications You can also search for this author inPubMed 

Google Scholar * John H Kersey View author publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE

THIS ARTICLE Filipovich, A., Spector, B. & Kersey, J. 806 IMMUNODEFICIENCY (ID) AS A SUSCEPTIBILITY FACTOR FOR MALIGNANCY IN ATAXIA-TELANGIECTASIA (A-T): REPORT FROM THE

IMMUNODEFICIENCY-CANCER REGISTRY (ICR). _Pediatr Res_ 15 (Suppl 4), 577 (1981). https://doi.org/10.1203/00006450-198104001-00831 Download citation * Issue Date: 01 April 1981 * DOI:

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